TABLE 1

Patient Characteristics and Organ Involvement

ParameterControlAAALATTRLocalized amyloidosis
Number of patients2060802722
Male:female14:619:4146:3413:1412:10
Age (y)53.3 (17.0)56.5 (14.7)61.8 (10.2)51.8 (10.5)50.6 (12.1)
I. Heart
 a. Mean thickness* > 12 mm1 (5%)7 (12%)36 (45%)9 (33%)0 (0%)
 b. Heart failure1 (5%)3 (5%)24 (30%)2 (7%)0 (0%)
 c. Low-voltage electrocardiography0 (0%)4 (7%)23 (29%)1 (4%)0 (0%)
 Heart involvement (a, b, or c)2 (10%)11 (18%)44 (55%)9 (33%)0 (0%)
II. Kidney
 d. Proteinuria > 0.5 g/24 h8 (40%)39 (65%)51 (64%)1 (4%)0 (0%)
 e. Creatinine clearance < 60 mL/min10 (50%)41 (68%)38 (48%)3 (11%)1 (5%)
 Kidney involvement (d or e)11 (55%)54 (90%)66 (83%)4 (15%)1 (5%)
III. Liver
 f. Liver span > 16 cm0 (0%)0 (0%)15 (19%)0 (0%)0 (0%)
 g. Alkaline phosphatase > 180 kU/L0 (0%)3 (5%)18 (23%)0 (0%)0 (0%)
 Liver involvement (f or g)0 (0%)3 (5%)24 (30%)0 (0%)0 (0%)
IV. Nervous tissue
 h. Peripheral polyneuropathy2 (10%)2 (3%)26 (33%)21 (78%)1 (5%)
 i. Autonomic neuropathy1 (5%)7 (12%)33 (41%)20 (74%)1 (5%)
 Nerve involvement (h or i)2 (10%)8 (13%)40 (50%)23 (85%)1 (5%)
Number of organs involved:
 06 (30%)4 (7%)2 (3%)2 (7%)20 (91%)
 112 (60%)41 (68%)20 (25%)15 (56%)2 (9%)
 22 (10%)10 (17%)27 (34%)9 (33%)0 (0%)
 30 (0%)5 (8%)24 (30%)1 (4%)0 (0%)
 40 (0%)0 (0%)7 (9%)0 (0%)0 (0%)
  • * Of cardiac septum and left ventricular wall.

  • According to New York Heart Association grades 3 or 4.

  • Data are mean, with SD in parentheses, or number, with percentage in parentheses.